Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia

Meiso Hayashi, Isabelle Denjoy, Fabrice Extramiana, Alice Maltret, Nathalie Roux Buisson, Jean Marc Lupoglazoff, Didier Klug, Miyuki Hayashi, Seiji Takatsuki, Elisabeth Villain, Joël Kamblock, Anne Messali, Pascale Guicheney, Joël Lunardi, Antoine Leenhardt

Research output: Contribution to journalArticle

278 Citations (Scopus)

Abstract

Background - The pathophysiological background of catecholaminergic polymorphic ventricular tachycardia is well understood, but the clinical features of this stress-induced arrhythmic disorder, especially the incidence and risk factors of arrhythmic events, have not been fully ascertained. Methods and Results - The outcome in 101 catecholaminergic polymorphic ventricular tachycardia patients, including 50 probands, was analyzed. During a mean follow-up of 7.9 years, cardiac events defined as syncope, aborted cardiac arrest, including appropriate discharges from implantable defibrillators, or sudden cardiac death occurred in 27 patients, including 2 mutation carriers with normal exercise tests. The estimated 8-year event rate was 32% in the total population and 27% and 58% in the patients with and without β-blockers, respectively. Absence of β-blockers (hazard ratio [HR], 5.48; 95% CI, 1.80 to 16.68) and younger age at diagnosis (HR, 0.54 per decade; 95% CI, 0.33 to 0.89) were independent predictors. Fatal or near-fatal events defined as aborted cardiac arrest or sudden cardiac death occurred in 13 patients, resulting in an estimated 8-year event rate of 13%. Absence of β-blockers (HR, 5.54; 95% CI, 1.17 to 26.15) and history of aborted cardiac arrest (HR, 13.01; 95% CI, 2.48 to 68.21) were independent predictors. No difference was observed in cardiac and fatal or near-fatal event rates between probands and family members. Conclusions - Cardiac and fatal or near-fatal events were not rare in both catecholaminergic polymorphic ventricular tachycardia probands and affected family members during the long-term follow-up, even while taking β-blockers, which was associated with a lower event rate. Further studies evaluating concomitant therapies are necessary to improve outcome in these patients.

Original languageEnglish
Pages (from-to)2426-2434
Number of pages9
JournalCirculation
Volume119
Issue number18
DOIs
Publication statusPublished - 2009 May 12
Externally publishedYes

Fingerprint

Heart Arrest
Incidence
Sudden Cardiac Death
Implantable Defibrillators
Syncope
Exercise Test
Polymorphic catecholergic ventricular tachycardia
Mutation
Population
Therapeutics

Keywords

  • Beta-blocker
  • Clinical genetics
  • Death
  • Follow-up studies
  • Mutation
  • Sudden
  • Tachycardia
  • Ventricular

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Cite this

Hayashi, M., Denjoy, I., Extramiana, F., Maltret, A., Buisson, N. R., Lupoglazoff, J. M., ... Leenhardt, A. (2009). Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia. Circulation, 119(18), 2426-2434. https://doi.org/10.1161/CIRCULATIONAHA.108.829267

Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia. / Hayashi, Meiso; Denjoy, Isabelle; Extramiana, Fabrice; Maltret, Alice; Buisson, Nathalie Roux; Lupoglazoff, Jean Marc; Klug, Didier; Hayashi, Miyuki; Takatsuki, Seiji; Villain, Elisabeth; Kamblock, Joël; Messali, Anne; Guicheney, Pascale; Lunardi, Joël; Leenhardt, Antoine.

In: Circulation, Vol. 119, No. 18, 12.05.2009, p. 2426-2434.

Research output: Contribution to journalArticle

Hayashi, M, Denjoy, I, Extramiana, F, Maltret, A, Buisson, NR, Lupoglazoff, JM, Klug, D, Hayashi, M, Takatsuki, S, Villain, E, Kamblock, J, Messali, A, Guicheney, P, Lunardi, J & Leenhardt, A 2009, 'Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia', Circulation, vol. 119, no. 18, pp. 2426-2434. https://doi.org/10.1161/CIRCULATIONAHA.108.829267
Hayashi M, Denjoy I, Extramiana F, Maltret A, Buisson NR, Lupoglazoff JM et al. Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia. Circulation. 2009 May 12;119(18):2426-2434. https://doi.org/10.1161/CIRCULATIONAHA.108.829267
Hayashi, Meiso ; Denjoy, Isabelle ; Extramiana, Fabrice ; Maltret, Alice ; Buisson, Nathalie Roux ; Lupoglazoff, Jean Marc ; Klug, Didier ; Hayashi, Miyuki ; Takatsuki, Seiji ; Villain, Elisabeth ; Kamblock, Joël ; Messali, Anne ; Guicheney, Pascale ; Lunardi, Joël ; Leenhardt, Antoine. / Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia. In: Circulation. 2009 ; Vol. 119, No. 18. pp. 2426-2434.
@article{af0f864e691543a8b87dc21c222fec10,
title = "Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia",
abstract = "Background - The pathophysiological background of catecholaminergic polymorphic ventricular tachycardia is well understood, but the clinical features of this stress-induced arrhythmic disorder, especially the incidence and risk factors of arrhythmic events, have not been fully ascertained. Methods and Results - The outcome in 101 catecholaminergic polymorphic ventricular tachycardia patients, including 50 probands, was analyzed. During a mean follow-up of 7.9 years, cardiac events defined as syncope, aborted cardiac arrest, including appropriate discharges from implantable defibrillators, or sudden cardiac death occurred in 27 patients, including 2 mutation carriers with normal exercise tests. The estimated 8-year event rate was 32{\%} in the total population and 27{\%} and 58{\%} in the patients with and without β-blockers, respectively. Absence of β-blockers (hazard ratio [HR], 5.48; 95{\%} CI, 1.80 to 16.68) and younger age at diagnosis (HR, 0.54 per decade; 95{\%} CI, 0.33 to 0.89) were independent predictors. Fatal or near-fatal events defined as aborted cardiac arrest or sudden cardiac death occurred in 13 patients, resulting in an estimated 8-year event rate of 13{\%}. Absence of β-blockers (HR, 5.54; 95{\%} CI, 1.17 to 26.15) and history of aborted cardiac arrest (HR, 13.01; 95{\%} CI, 2.48 to 68.21) were independent predictors. No difference was observed in cardiac and fatal or near-fatal event rates between probands and family members. Conclusions - Cardiac and fatal or near-fatal events were not rare in both catecholaminergic polymorphic ventricular tachycardia probands and affected family members during the long-term follow-up, even while taking β-blockers, which was associated with a lower event rate. Further studies evaluating concomitant therapies are necessary to improve outcome in these patients.",
keywords = "Beta-blocker, Clinical genetics, Death, Follow-up studies, Mutation, Sudden, Tachycardia, Ventricular",
author = "Meiso Hayashi and Isabelle Denjoy and Fabrice Extramiana and Alice Maltret and Buisson, {Nathalie Roux} and Lupoglazoff, {Jean Marc} and Didier Klug and Miyuki Hayashi and Seiji Takatsuki and Elisabeth Villain and Jo{\"e}l Kamblock and Anne Messali and Pascale Guicheney and Jo{\"e}l Lunardi and Antoine Leenhardt",
year = "2009",
month = "5",
day = "12",
doi = "10.1161/CIRCULATIONAHA.108.829267",
language = "English",
volume = "119",
pages = "2426--2434",
journal = "Circulation",
issn = "0009-7322",
publisher = "Lippincott Williams and Wilkins",
number = "18",

}

TY - JOUR

T1 - Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia

AU - Hayashi, Meiso

AU - Denjoy, Isabelle

AU - Extramiana, Fabrice

AU - Maltret, Alice

AU - Buisson, Nathalie Roux

AU - Lupoglazoff, Jean Marc

AU - Klug, Didier

AU - Hayashi, Miyuki

AU - Takatsuki, Seiji

AU - Villain, Elisabeth

AU - Kamblock, Joël

AU - Messali, Anne

AU - Guicheney, Pascale

AU - Lunardi, Joël

AU - Leenhardt, Antoine

PY - 2009/5/12

Y1 - 2009/5/12

N2 - Background - The pathophysiological background of catecholaminergic polymorphic ventricular tachycardia is well understood, but the clinical features of this stress-induced arrhythmic disorder, especially the incidence and risk factors of arrhythmic events, have not been fully ascertained. Methods and Results - The outcome in 101 catecholaminergic polymorphic ventricular tachycardia patients, including 50 probands, was analyzed. During a mean follow-up of 7.9 years, cardiac events defined as syncope, aborted cardiac arrest, including appropriate discharges from implantable defibrillators, or sudden cardiac death occurred in 27 patients, including 2 mutation carriers with normal exercise tests. The estimated 8-year event rate was 32% in the total population and 27% and 58% in the patients with and without β-blockers, respectively. Absence of β-blockers (hazard ratio [HR], 5.48; 95% CI, 1.80 to 16.68) and younger age at diagnosis (HR, 0.54 per decade; 95% CI, 0.33 to 0.89) were independent predictors. Fatal or near-fatal events defined as aborted cardiac arrest or sudden cardiac death occurred in 13 patients, resulting in an estimated 8-year event rate of 13%. Absence of β-blockers (HR, 5.54; 95% CI, 1.17 to 26.15) and history of aborted cardiac arrest (HR, 13.01; 95% CI, 2.48 to 68.21) were independent predictors. No difference was observed in cardiac and fatal or near-fatal event rates between probands and family members. Conclusions - Cardiac and fatal or near-fatal events were not rare in both catecholaminergic polymorphic ventricular tachycardia probands and affected family members during the long-term follow-up, even while taking β-blockers, which was associated with a lower event rate. Further studies evaluating concomitant therapies are necessary to improve outcome in these patients.

AB - Background - The pathophysiological background of catecholaminergic polymorphic ventricular tachycardia is well understood, but the clinical features of this stress-induced arrhythmic disorder, especially the incidence and risk factors of arrhythmic events, have not been fully ascertained. Methods and Results - The outcome in 101 catecholaminergic polymorphic ventricular tachycardia patients, including 50 probands, was analyzed. During a mean follow-up of 7.9 years, cardiac events defined as syncope, aborted cardiac arrest, including appropriate discharges from implantable defibrillators, or sudden cardiac death occurred in 27 patients, including 2 mutation carriers with normal exercise tests. The estimated 8-year event rate was 32% in the total population and 27% and 58% in the patients with and without β-blockers, respectively. Absence of β-blockers (hazard ratio [HR], 5.48; 95% CI, 1.80 to 16.68) and younger age at diagnosis (HR, 0.54 per decade; 95% CI, 0.33 to 0.89) were independent predictors. Fatal or near-fatal events defined as aborted cardiac arrest or sudden cardiac death occurred in 13 patients, resulting in an estimated 8-year event rate of 13%. Absence of β-blockers (HR, 5.54; 95% CI, 1.17 to 26.15) and history of aborted cardiac arrest (HR, 13.01; 95% CI, 2.48 to 68.21) were independent predictors. No difference was observed in cardiac and fatal or near-fatal event rates between probands and family members. Conclusions - Cardiac and fatal or near-fatal events were not rare in both catecholaminergic polymorphic ventricular tachycardia probands and affected family members during the long-term follow-up, even while taking β-blockers, which was associated with a lower event rate. Further studies evaluating concomitant therapies are necessary to improve outcome in these patients.

KW - Beta-blocker

KW - Clinical genetics

KW - Death

KW - Follow-up studies

KW - Mutation

KW - Sudden

KW - Tachycardia

KW - Ventricular

UR - http://www.scopus.com/inward/record.url?scp=66549094345&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=66549094345&partnerID=8YFLogxK

U2 - 10.1161/CIRCULATIONAHA.108.829267

DO - 10.1161/CIRCULATIONAHA.108.829267

M3 - Article

C2 - 19398665

AN - SCOPUS:66549094345

VL - 119

SP - 2426

EP - 2434

JO - Circulation

JF - Circulation

SN - 0009-7322

IS - 18

ER -